Eye (1991) S, 64 9-661 Fuchs' Heterochromic Uveitis: A Reappraisal of the Clinical Spectrum NICHOLAS P. JONES Manchester Summary The varying clinical appearance within a group of 103 patients with Fuchs' Hetero­ chromic Uveitis (FHU) is discussed. Bilateral cases accounted for 7.8% of patients. Cataract was found in 80.2% of cases after 8.8 years mean follow-up, and glaucoma affected 26.2% of patients. The most important diagnostic criteria are the typical pattern of iris atrophy and depigmentation, the characteristic intraocular inflam­ mation, and complicated cataract. These are described in detail. The correct diag­ nosis of FHU is important because it affects future management and prognosis. The presence or absence of macroscopic heterochromia is not a helpful component of the ocular examination and should play little part in diagnosis. FHU does not necessarily present in its classical form, and may first resemble acute anterior uveitis or pars planitis. The syndrome may be the end stage of a number of different conditions. Although the combination of heterochromia tis (FHU) has placed into perspective some of and cataract was noted in 1843 by Lawrence, I the accepted criteria for diagnosis, has and later by others2,3 it was not until 1906 that allowed further comment on some aspects of Ernst Fuchs comprehensively described a previous reports, and has brought to light group of 38 patients with signs of a form of some features not previously discussed in heterochromia with inflammation and catar­ detaiL The clinical spectrum of FHU is wider act, dubbed 'complicated heterochromia',4 than has been previously defined, and the The syndrome which now bears his name has clinical course is more variable, Studies on a interested many authors in the succeeding 85 cohort of 103 patients with FHU are reported. years and some series of considerable size have been published,5-8, The classical diag­ nostic criteria for the condition were reiter­ Patients and Methods ated in 1955 by Franceschetti5 and include the Commencing in 1987, all new and old patients presence of stellate keratic precipitates, a seen at the Manchester Royal Eye Hospital characteristic pattern of iris atrophy, with or with a definiteor suggested diagnosis of FHU, without nodules (and without posterior syn­ were referred to and examined by the author, echiae), vitreous opacification, cataract and In all cases a detailed history was taken, possibly glaucoma, A comprehensive review9,10 including mode of, and reason for, presen­ of the literature was published in 1973. tation, General medical, neonatal, gesta­ The critical examination of a large number tional and family histories were included. The of patients with Fuchs' Heterochromic Uvei- clinical course of the disease process was Correspondence to: Mr. N. P. Jones, FRCS, FCOphth, University Department of Ophthalmology, Manchester Royal Eye Hospital, Oxford Road, Manchester M13 9WH. 650 N. P. JONES noted, including a record of medical and sur­ distinct groups; fifteen patientshad congeni­ gical management. tal heterochromia and 25 had noticed a later A full clinical examination included onset. The age at which the heterochromia appraisal of facial appearance, slit-lamp bio­ was firstnoticed, for the 25 patients in which it microscopy including a critical examination of was not congenital, is shown in Fig. 2). cornea, anterior chamber and iris, applana­ A previous ophthalmic history revealed tion, and dilatation for fundoscopy. Corneal that 16 patients (15.5%) had had poor vision endothelial specular microscopy and haemat­ in the affected eye since childhood, but none ological investigations were performed in had presented to an ophthalmologist at that selected cases. Most patients have been exam­ stage. A lower best visual acuity on the ined on several occasions by the author. A affectedside (in those with clear media and no total of 128 patients were referred with a defi­ macular disease) implied amblyopia. There nite or suggested diagnosis of FHU. Of these, was no history of strabismus in any of these 103 were considered to fulfil criteria for this patients. Only three patients had significant diagnosis and were included in the survey. anisometropia, the affected eye being more myopic in all three cases. No patient had evi­ Results dence of amblyopia in the eye without inflam­ The patients' ages at presentation are shown mation. Of these patients with amblyopia, graphically in Fig. 1. The range was 8 to 71 nine were aware of their heterochromia, and years, with a mean of 36. 1 years. The major in four cases it was definitelyacquired, at ages symptoms at presentation are shown in 17, 21, 35 and 38 years, respectively. Two Table 1. The follow-up since presentation has patients remembered a blunt anjury of some a range of six months to 59 years, with a mean significance to the involved eye at some time of 8.8 years. Fifty patients (48.5%) were male previously. Nineteen patients complained of and 53 (51.5%) female. Ninety-five patients eye or brow ache before or since presentation. (92.2%) were Caucasian, three Asian and five Three patients were aware of a significant Afro-caribbean or mixed race (total 7.8% maternal illness during pregnancy. One non-Caucasian). The precise catchment mother had serum hepatitis, one had epileptic population of this hospital cannot be defined, fits (the reason being unknown), and another but the racial balance of these patients prob­ required transfusion. Ninety-eight patients ably does not indicate any racial predilection were delivered vaginally at term without inci­ for the disease. dent. Five patients were delivered with The diagnosis of FHU was not always made forceps, of whom one was known to have sus­ immediately on presentation. The delay in tained an ocular injury, and one claimed to diagnosis ranged from 0 to 57 years, with a have been injured. The only significant prob­ mean of 3.4 years. Table II shows the diag­ lem in the neonatal period involved one noses made on presentation. patient with a ventricular septal defect which The degree of heterochromia was graded required closure. 0-4 by the author. Heterochromia was absent In no patient in this series was evidence (Grade 0) in 10 patients, of whom eight found of a positive familial association for (7.8%) had bilateral disease and two had dark FHU or any other variety of heterochromia. brown irides. Heterochromia Grade 1-4 was On the contrary, discordance in a pair of present in the remaining 93 patients (90.3%) monozygotic twins has been discovered. The but was extremely subtle (Grade 1) in 42 implications of this on any genetic theory for cases, though usually still apparent with the FHU are discussed elsewhere. II naked eye. In a small number of patients, het­ Enquiries into the general health of these erochromia could only be definedby compari­ patients revealed the following; three patients son of anterior segment photographs. have peptic ulceration, two systemic hyper­ Of those patients with heterochromia, 40 tension, and one each have Addison's (43.0% ) were already aware of this fact. All of disease, hypernephroma, diabetes, epilepsy, these patients knew the time of onset of their pernicious anaemia, Parkinsonism, ischaemic heterochromia, and appeared to fall into two heart disease, discoid lupus erythematosus, FUCHS' HETEROCHROMIC UVEITIS: A REAPPRAISAL OF THE CLINICAL SPECTRUM 651 2 NUMBER OF PATIENTS acellular on specular microscopy, and one cor­ 0 nea showed linear marks in Descemet's mem­ brane typical of previous trauma and r- 5 consistent with a history of forceps damage ,- � during delivery. c- Keratic precipitates (KP) were present in 0 o- 93 eyes (83.8%). The distribution of precip­ r- � itates was variable, yet seemed to fitinto three r 1 broad patterns (Fig. 3); generalised, affecting the whole posterior cornea (57 eyes); central nn I In II 1-1'1 (16 eyes); and inferior (20 eyes), often dif­ -5 -10 -15 -20 -25 -30 -35 -40 -45 -50 -55 -60 -65 -70 -75 fusely so but occasionally in a triangular distri­ AGE AT PRESENTATION bution more commonly associated with acute Fig. 1. The age range of patients with FHU at first presentation to an ophthalmologist. anterior uveitis. The morphology of KP was variable, but many eyes (45, 40.5%) showed previous tuberculosis, and Factor VII defi­ precipitates previously described as typical of ciency. One patient is a haemophilia carrier. FHU,5.12 that is, stellate with fibrillary exten­ On examination, the right eye was affected sions, and tiny interspersed fibrils. A dia­ in 50 cases (48.5%) and the left in 45 (43.6%), grammatic example of a typical area of such the remaining eight patients being bilateral. keratic precipitates is shown in Fig. 4. Nine­ Head or facial appearance was notable in only teen eyes (17. 1%) had KP which are two cases, both of whom were affected by the described as 'glassy', being medium-sized, Parry-Romberg syndrome of hemifacial atro­ globular, transparent and non-pigmented. phy on the affected side. Ten patients had a Old pigmented KP were seen in five eyes. partial ptosis on the affected side, of whom There was some correlation between mor­ eight had undergone cataract surgery. No phology of KP and their distribution. Typi­ patient in this series had acceptable evidence cally stellate KP were centrally or generally distributed (38 of 45 eyes, 84.4%). Only seven of Homer's syndrome. The cornea of the affec ed eye, examined of 20 eyes (35%) with inferiorly-distributed . � with the slit-lamp biomicroscope, was KP showed stellate characteristics. Anterior chamber activity was recorded abnormal in 17 eyes (15.3%) of whom eight + + +. had previously undergone cataract surgery. traditionally on a scale of 0 to Fifty­ nine eyes (53. 1 %) had no cellular activity and Three corneas were partially or completely (+).